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Rabbit Anti-COX3/BF350 Conjugated antibody (bs-3934R-BF350)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號 bs-3934R-BF350
英文名稱1 Rabbit Anti-COX3/BF350 Conjugated antibody
中文名稱 BF350標(biāo)記的細(xì)胞色素C氧化酶亞基3抗體
別    名 Cytochrome c oxidase polypeptide III; Cytochrome c oxidase subunit 3; COIII; COX3; COX-3; COXIII; MT CO3; MTCO3; COX3_HUMAN; mitochondrially encoded cytochrome c oxidase III; MT-CO3.  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 腫瘤  細(xì)胞生物  免疫學(xué)  神經(jīng)生物學(xué)  信號轉(zhuǎn)導(dǎo)  轉(zhuǎn)錄調(diào)節(jié)因子  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Mouse, Rat,  (predicted: Human, Chicken, Dog, Pig, Cow, Horse, Rabbit, )
產(chǎn)品應(yīng)用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 30kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human COX3
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產(chǎn)品介紹 Function:
Subunits I, II and III form the functional core of the enzyme complex.

Subcellular Location:
Mitochondrion inner membrane; Multi-pass membrane protein.

DISEASE:
Leber hereditary optic neuropathy (LHON) [MIM:535000]: A maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes. Note=The disease is caused by mutations affecting the gene represented in this entry.
Mitochondrial complex IV deficiency (MT-C4D) [MIM:220110]: A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, exercise intolerance, developmental delay, delayed motor development and mental retardation. Some affected individuals manifest a fatal hypertrophic cardiomyopathy resulting in neonatal death. A subset of patients manifest Leigh syndrome. Note=The disease is caused by mutations affecting the gene represented in this entry.
Recurrent myoglobinuria mitochondrial (RM-MT) [MIM:550500]: Recurrent myoglobinuria is characterized by recurrent attacks of rhabdomyolysis (necrosis or disintegration of skeletal muscle) associated with muscle pain and weakness, and followed by excretion of myoglobin in the urine. Note=The gene represented in this entry may be involved in disease pathogenesis.

Similarity:
Belongs to the cytochrome c oxidase subunit 3 family.

Database links:

Entrez Gene: 281921 Cow

Entrez Gene: 4514 Human

Entrez Gene: 17710 Mouse

Entrez Gene: 26204 Rat

Omim: 516050 Human

SwissProt: P00415 Cow

SwissProt: P00414 Human

SwissProt: P00416 Mouse

SwissProt: P05505 Rat




Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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